Idiocy is an innate, the most severe form of mental retardation. Pathology begins to manifest itself from the first weeks of a child’s life and is expressed in a sharp lag in psychomotor development. Such patients lack speech and any other forms of mental activity, they are absolutely helpless and cannot master even the most primitive skills. The emotional background is also not developed, such patients do not recognize relatives and friends. The diagnosis is established on the basis of anamnesis, early manifestation of pathology and assessment of mental functions. Treatment is prescribed pathogenetic (enzyme therapy, hormonal treatment, anti-infective) and symptomatic (sedatives, nootropics, neuroleptics, etc.).
F73 Deep mental retardation
Idiocy (from the Greek idioteia, meaning “lack of learning” or idios–existing for oneself), is a deep form of mental retardation, the coefficient of intellectual development or IQ of such patients is less than 20 (less often from 20 to 35). The total number of patients with this diagnosis is equal to 3-5% of the total number of patients with oligophrenia (mental retardation). The prevalence among the population is approximately 1 case per 10,000. A rather rare, progressive pathology; manifesting from the first months of life. The disease is characterized by a rapid course, pronounced disorders of mental functions, severe lesions of the internal organs.
All the variety of etiological factors of idiotic development can be divided into endogenous-hereditary (occurring most often) and exogenous influences (noted less often) at the time of conception and on the fetus during pregnancy (embryo and fetopathy).
The main causes are gene abnormalities, chromosomal mutations (Down’s disease, cat’s cry syndrome, Shereshevsky-Turner and Kleifelter’s disease), recessive-inherited metabolic forms of congenital dementia (phenylketonuria, gargoilism, galactosemia, etc.). Also, idiocy can cause endogenous hereditary pathologies, which include craniostenosis, congenital anomaly brain, microcephaly.
Exogenous influences also contribute to the development of idiocy. The most common is an infectious factor. The pathological effect on the fetus of infectious diseases such as rubella, toxoplasmosis, cytomegalovirus infection, listeriosis is possible due to the penetration of viruses and bacteria through the fetoplacental barrier. Less important is the syphilis of the parents.
A special role is played by toxic effects on the embryo and fetus, namely alcoholism of parents (especially mothers), taking certain medications by the mother during pregnancy (antibiotics, barbiturates, sulfonamide preparations, and others). The incompatibility of the fetus and the mother according to the ABO system and the Rh factor is also important.
Pathological processes and traumas in childbirth and early postnatal development cannot cause the development of idiocy, but they can aggravate the current clinical situation (asphyxia in childbirth, damage to the brain itself, intracranial hematomas, intracranial infections).
Manifestations of pathology begin to be noticeable already in the first half of the child’s life. The earliest signs of idiocy are the absence or weakness of reactions to the environment, the absence of a differential smile, a complex of revival when relatives approach. There is an inability to distinguish loved ones from strangers; there is no reaction to the mother, interest in toys, expressive mimic activity – all this is a characteristic clinical manifestation of congenital dementia. The child has an expressionless look, a smile appears late, there is no subject-manipulative activity and an initial understanding of the reversed speech. The degree of lag in motor development begins to manifest itself more and more clearly over time.
At an older age, the main clinical manifestations of the disease are the lack of speech and underdevelopment of other mental functions. It is difficult to come into contact with patients, since they do not perceive the speech addressed to them, the reaction to the environment either does not occur at all, or has a perverted, inadequate character. Attention is unstable or completely absent. Such patients have completely undeveloped motor and static functions; they are unable to serve themselves and do not possess even the simplest self-service skills, and therefore need constant supervision and care.
Only the most elementary mental operations are available to patients, and their mental life is at the level of unconditionally reflex (a conditioned reflex can only be formed for feeding). Basic mental functions are either completely absent or underdeveloped. Emotions are not differentiated and are represented only by two opposite reactions – pleasure and discontent. Patients are voracious, they pull absolutely everything into their mouths – edible or inedible.
There are two main clinical forms of idiocy – torpid and excitable. In the torpid variant, patients are left to themselves and remain motionless; in the excitable one, they are in a state of constant non-directional and often stereotypical psychomotor agitation (clap their hands, sway and make other movements).
Quite often, a severe form of mental retardation is combined with anomalies and malformations of physical development. They can be represented by various dysplasias: defects in the development of the upper and lower extremities, six-fingered or finger fusion, congenital contractures of the joints, spinal and cerebral hernias; diastema, underdevelopment or defects of the outer ear are also quite common. Malformations of internal organs are also frequent: congenital heart defects, dysgenesis of the gastrointestinal tract, malformations of the genitourinary system.
The diagnosis is made by a pediatrician or neurologist on the basis of clinical research and observation of the patient. The neurologist evaluates the mental and emotional state of the patient, thinking, reflexes, the degree of intelligence development. If necessary, CT or MRI of the brain is performed in order to detail the damage.
Differential diagnosis of idiocy is carried out with other forms of organic brain damage (schizophrenia, brain injury, vascular diseases). Pathology is distinguished from the latter by the total nature of the disease, affecting not only the actual thought processes, but also perception, memory, attention, emotional and motor-volitional spheres of mental activity.
Given the innateness and hereditary nature of the development of pathology, it is not possible to cure idiocy. In this regard, pathogenetic therapy is prescribed: for enzymopathies – replenishment of the lack of enzymes, for endocrinopathies – hormonal correction of the latter; specific treatment is prescribed for congenital syphilis and toxoplasmosis.
Symptomatic treatment includes dehydration (with increased intracranial pressure, this is magnesia, acetazolamide and other diuretics), restorative (vitamins) and sedative therapy. Drugs of metabolic action are prescribed, which to some extent contribute to the restoration of mental functions (gamma-aminobutyric acid, cinnarizine, piracetam, pyritinol and others).
In the torpid clinical form, stimulants are used (mesocarb, ginseng, Chinese lemongrass, aloe, etc.). In the excitable form of neuroleptics; in the presence of epi-seizures, anticonvulsants. The purpose of physical therapy (physical therapy) is shown. Such patients require constant care and supervision.
Primary prevention includes medical and genetic counseling, adequate management of pregnancy and early diagnosis of abnormal fetal development, careful prescription of medications to a pregnant woman, her release from work related to occupational hazards, complete abstinence from alcohol and smoking, starting from the moment of pregnancy planning.
Secondary prevention consists in early detection of idiocy and timely treatment and necessary rehabilitation measures.